Babinski

RATIONALE: X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene leading to very long chain fatty acid (VLCFA) accumulation. The disease demonstrates a spectrum of phenotypes including adrenomyeloneuropathy (AMN). We aimed to identify the genetic basis of disease in a patient presenting with AMN features in order to confirm the diagnosis, expand genetic knowledge of ABCD1 mutations, and elucidate potential genotype-phenotype associations to inform management. PATIENT CONCERNS: A 29-year-old male presented with a 4-year history of progressive spastic paraplegia, weakness of lower limbs, fecal incontinence, sexual dysfunction, hyperreflexia, and positive Babinski and Chaddock signs...

BACKGROUND: Sarcoidosis, a multisystem inflammatory non-caseating granulomatous disease, can present with neurologic lesions in up to 10% of patients. CASE DESCRIPTION: A 57-year-old male presented with three months of worsening upper extremity radicular pain associated with dysmetria, hyperreflexia, bilateral Hoffman's, and positive Babinski signs. The contrast magnetic resonance imaging (MRI) showed a diffuse T2 signal hyperintensity and T1-enhancing 2.5 cm lesion extending sagittally between C4 and C6...

Anton-Babinski syndrome is a rare extension of cortical blindness following injury to the occipital lobe. The patient behaves as sighted but has visual function derangements. The posterior cerebral artery (PCA) stroke represents 5% to 10% of total strokes. The COVID-19 pandemic has shown a rise in stroke cases. We present a case of this rare PCA stroke, first diagnosed by an ophthalmologist. This case had an inconsistent initial presentation, but subsequent computed tomography of the brain and other neurological investigations confirmed the diagnosis...

The deep tendon reflex (DTR) is a more objective indicator than sensory and muscle assessments for lumbar spine disorders. Further, unlike sensory and muscle assessments that require patient cooperation, the DTR can be assessed even in patients with impaired consciousness or cognition. Therefore, DTR assessment with a hammer is an essential neurological test for lumbar spinal diseases. However, despite the usefulness of DTR assessment, few reports have described the significance of increased, diminished, or absent deep lower extremity reflexes in lumbar spine diseases...

INTRODUCTION: Cervical disc herniation, which often results in ipsilateral upper extremity pain corresponding with the side of herniation, is rarely reported to cause contralateral radiculopathy. CASE PRESENTATION: A 53-year-old man presented to our hospital with left upper arm pain radiating to his left hand. On physical examination, there was hypesthesia in the left thumb, index, and middle finger. Muscle strength was 4 in the left arm and 5 in the other extremities...

The efficacy and optimal frequency of acupuncture for hemifacial spasms (HFSs) in patients unresponsive or averse to standard treatment methods remains unestablished. Here, we administered acupuncture to a patient with HFSs who was dissatisfied with the outcomes of botulinum toxin (BoNT) injections as symptomatic treatment. A man in his 60s, experiencing frequent spasms in his left facial muscles since 2015, had received several BoNT injections without receiving microvascular decompression or medication; however, the treatment results were not satisfactory...

INTRODUCTION: Numerous reflexive responses have been documented as alterations to the Babinski sign within upper motor neuron lesions. However, scant attention has been given to reflexes beyond these, which exhibit independence from the extensor plantar response. These reflexes predominantly form polysynaptic arcs, with nociceptive stimuli acting as afferents. CASE REPORT: The reflex was serendipitously discovered in an 18-year-old female patient who presented with spastic paraplegia with bowel and bladder involvement, as a consequence of an aneurysmal bone cyst of the D3 (dorsal) vertebrae, and the same was named after the authors as "Yadav-Kunal reflex" which can be defined as: "In individuals with spastic paraparesis, forcibly plantarflexing the toes will result in sudden jerky flexion of the knee and hip on the same side...

We investigate the vibrational and magnetic properties of thin layers of chromium tribromide (CrBr3 ) with a thickness ranging from three to twenty layers (3-20 L) revealed by the Raman scattering (RS) technique. Systematic dependence of the RS process efficiency on the energy of the laser excitation is explored for four different excitation energies: 1.96 eV, 2.21 eV, 2.41 eV, and 3.06 eV. Our characterization demonstrates that for 12 L CrBr3 , 3.06 eV excitation could be considered resonant with interband electronic transitions due to the enhanced intensity of the Raman-active scattering resonances and the qualitative change in the Raman spectra...

Friedreich's ataxia is degenerative disease frequently starting around puberty and it's characterized by a progressive gait ataxia, limb weakness, apparition of Babinsky sign, loss of deep tendon reflex, dysarthria and skeletal deformities. The development of vestibular pathology is common but not completely understood. A 16 years old woman with early vestibular defects in relation to a latter Friedreich's ataxia diagnosis is reported.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a rare neurological disorder characterized by inflammation in the brain and spinal cord. This systematic review aims to investigate the potential association between ADEM and influenza vaccination by analyzing relevant case reports. ADEM is traditionally thought to be a monophasic condition, predominantly affecting children, often following viral illnesses or immunizations. Recent attention has focused on a possible link between ADEM and influenza vaccination, prompting the need for a thorough investigation...

The optical response of two-dimensional (2D) perovskites, often referred to as natural quantum wells, is primarily governed by excitons, whose properties can be readily tuned by adjusting the perovskite layer thickness. We have investigated the exciton fine structure splitting in the archetypal 2D perovskite (PEA)2 (MA) n -1 Pb n I3 n +1 with varying numbers of inorganic octahedral layers n = 1, 2, 3, and 4. We demonstrate that the in-plane excitonic states exhibit splitting and orthogonally oriented dipoles for all confinement regimes...

PURPOSE OF REVIEW: In childhood, attention-deficit/hyperactivity disorder (ADHD) is diagnosed three to 16 times more frequently in males compared to females, yet in adulthood, nearly equivalent numbers of males and females are diagnosed with ADHD. Relatively few studies have prioritized examination of sex differences in ADHD even though sex differences may have critical implications for the identification and treatment of ADHD in females and males. This review highlights current research on sex differences in ADHD across the lifespan that has emerged from cross-sectional and prospective longitudinal studies of youth, adult-ascertained samples, and registry studies...

Moiré excitons (MXs) are electron-hole pairs localised by the periodic (moiré) potential forming in two-dimensional heterostructures (HSs). MXs can be exploited, e.g., for creating nanoscale-ordered quantum emitters and achieving or probing strongly correlated electronic phases at relatively high temperatures. Here, we studied the exciton properties of WSe2 /MoSe2 HSs from T = 6 K to room temperature using time-resolved and continuous-wave micro-photoluminescence also under a magnetic field. The exciton dynamics and emission lineshape evolution with temperature show clear signatures that MXs de-trap from the moiré potential and turn into free interlayer excitons (IXs) for temperatures above 100 K...

This study aims to summarize the clinical characteristics and prognosis of Listeria monocytogenes (LM) meningitis in children in Chongqing, China. A retrospective analysis of the clinical data and follow-up results of 20 LM meningitis patients admitted to the Children's Hospital of Chongqing Medical University (CHCMU) from January 2012 to December 2022 was performed. The LM meningitis incidence rate was between 0 and 14.3 per 100,000 persons. The median age at onset was 8.98 months. There were five neonate cases, which all had perinatal abnormalities...

INTRODUCTION: Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes. MATERIALS AND METHODS: The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations...

Facial spasm is a disorder characterized by mostly unilateral(hemifacial)involuntary facial muscle contractions, usually caused by vascular compression of the facial nerve. It has been known since ancient times and we can currently find both old medical and artistic presentations. Charles Bell has described at least one definite case(No. IV)with hemifacial spasm in his textbook published in 1830 as did the following physicians like Romberg, Hammond, Gowers, and Brissaud. Babinski coined the name hemispasme facial in 1905 and its English term "hemifacial spasm" appeared in the article by Ehni et al...

PURPOSE: Multiple Sclerosis (MS) is a neuro-inflammatory disease of the Central Nervous System (CNS) in which the body's immune system attacks and destroys myelin sheath that protects nerve fibers and causes disruption in axonal signal transmission. Demyelinating Optic Neuritis (ON) is often a manifestation of MS and involves inflammation of the optic nerve. ON can cause vision loss, pain and discomfort in the eyes, and difficulties in color perception.In this study, we developed NeuroVEP, a portable, wireless diagnostic system that delivers visual stimuli through a smartphone in a headset and measures evoked potentials at the visual cortex from near the O1, Oz, O2, O9 and O10 locations on the scalp (extended 10-20 system) using custom electroencephalography (EEG) electrodes...

Hypertrophic pachymeningitis (HP) is a rare presentation with duramater thickening and fibrosis which can result in cranial or spinal compressive disease. Most cases of spinal HP require surgical management. We present an uncommon case of idiopathic hypertrophic spinal pachymeningitis (IHSP) in a 40-year-old male who showed complete improvement to steroids without any further relapses. The patient presented with bilateral upper limb weakness with magnetic resonance imaging (MRI) spine showing diffuse dural thickening of the entire spine with cervical cord compression...

Coffee, black with a spritz of milk. I enjoyed routine, and today was no different. We were on the 12th floor of the "tower", as our hospital is lovingly called. It was my second year of residency, and I was charged with admissions to our neurological ward. Sighing with the expectation of a long day, I walked over to our patients. The first was a woman in her sixties. She complained of trouble swallowing for the past 2 months that was getting worse. She was otherwise healthy. Asked about her social background, she looked over to her husband with an impish grin and replied: "happily married for almost thirty years...

Cerebellar pial arteriovenous fistula (PAVF) in adults presenting with congestive edema of the brainstem and cervical cord has rarely been reported. Here, we report such a case. A 59-year-old man presented with progressive weakness and numbness of the limbs and hiccups for 6 months. On physical examination, his limbs had grade III muscle strength, and he was unable to stand or walk. He also had occasional incontinence and retention. There was a positive Babinski sign in both lower limbs. Below the neck, he had hypoesthesia of the skin...